Giant Cell Tumor of Tendon Sheath



Giant Cell Tumor of Tendon Sheath


David R. Lucas, MD









Giant cell tumor of tendon sheath (GCTTS) typically presents as a painless, slow-growing mass that arises from a tendon sheath, most often on the volar aspect of a finger, such as this thumb mass image.






GCTTS has a mixture of mononuclear stromal cells, multinucleated giant cells image, and macrophages, including xanthoma cells image. Stromal fibrosis image and hemosiderin deposits image are very common.


TERMINOLOGY


Abbreviations



  • Giant cell tumor of tendon sheath (GCTTS)


Synonyms



  • Localized tenosynovial giant cell tumor, localized pigmented villonodular synovitis (PVNS), nodular tenosynovitis


Definitions



  • Benign soft tissue tumor of synovial origin



    • Polymorphous population of neoplastic stromal cells, macrophages, and osteoclast-like giant cells


    • Well circumscribed, noninvasive


ETIOLOGY/PATHOGENESIS


Histogenesis



  • Neoplastic growth



    • Balanced translocation involving 1p13 (CSF1 gene) in many tumors



      • CSF1 overexpression by neoplastic stromal cells


      • Recruitment and activation of intratumoral macrophages by CSF1R activation


CLINICAL ISSUES


Epidemiology



  • Incidence



    • 2nd most common tumor of hand


  • Age



    • Any age, peak 3rd-4th decade


  • Gender



    • Women outnumber men 2:1


Site



  • Digits (85%)



    • Especially fingers (75%)


    • Tendon sheath (usually volar) or interphalangeal joint


  • Large joints (10%)



    • Ankle, knee, wrist, elbow


    • Bursa


    • Intraarticular tumors called localized PVNS


Presentation



  • Painless mass


  • Slow growing


  • Uncommon findings: Triggering, carpal and ulnar tunnel syndromes


Treatment



  • Surgical approaches



    • Complete local excision


Prognosis



  • Benign but recurs locally (around 20%)


  • Risk factors for recurrence: Degenerative joint disease, distal phalanx, interphalangeal joint of thumb, osseous erosion

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Giant Cell Tumor of Tendon Sheath
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